Volume 5 Issue 1

A lot of research has been performed all over the world on umbilical cord blood (UCB), and many UCB banks have been instituted in the U.S.A. and Europe, since Gabutti, for the first time, collected and isolated several foetal haematopoietic progenitor cells from the residual placental blood, after delivery and ligation of the umbilical cord. In Italy, also, UCB banks belonging to the GRACE group of Milan connected to the European Netcord have been established. Recently, a new UCB bank has been instituted in Sicily, particularly in Sciacca.

Noonan Syndrome (NS) is an autosomal dominant disorder with complete penetrance and variable expressivity that affects 1:1000 to 1:2500 live births [1]. It is characterized by facial dysmorphism, short stature, heart defects, skeletal malformations, bleeding diatheses, and mild mental delay. Congenital cardiac defects are encountered in more than 80% of cases, with the majority of cardiac defects being pulmonary stenosis and hypertrophic cardiomyopathy [2]. Literature remains sparse with regards to management of NS in pregnancy.

Tuberous Sclerosis Complex (TSC) is an autosomal dominant disorder characterized by benign tumors in multiple organs, most frequently the skin and kidneys. This is associated with functional loss of tumor suppressors TSC1 and TSC2. Angiomyolipomas (AMLs) are usually slow-growing hamartomas rich in muscle, fat, and blood vessels that are present in approximately 80% of patients with TSC . These tumors exhibit high-grade mitotic activity, pleomorphism, and necrosis , making them susceptible to rupture and hemorrhage . While distribution of angiomyolipomas has been well described in nervous, renal, hepatic, cardiovascular, and integumentary systems, conspicuously few cases of angiomyolipoma involving the uterus have been reported in this patient population.